Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy

Three patients with the clinical and investigation features of facial onset sensory and motor neuronopathy (FOSMN) syndrome are presented, one of whom came to a post-mortem examination. This showed TDP-43-positive inclusions in the bulbar and spinal motor neurones as well as in the trigeminal nerve...

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Main Authors:	Ziso, Besa, Williams, Tim L., Walters, R. Jon L., Jaiser, Stephan R., Attems, Johannes, Wieshmann, Udo C., Larner, A.J., Jacob, Anu
Format:	Online
Language:	English
Published:	S. Karger AG 2015
Online Access:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4448067/

id	pubmed-4448067
recordtype	oai_dc
spelling	pubmed-44480672015-08-31 Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy Ziso, Besa Williams, Tim L. Walters, R. Jon L. Jaiser, Stephan R. Attems, Johannes Wieshmann, Udo C. Larner, A.J. Jacob, Anu Published online: April, 2015 Three patients with the clinical and investigation features of facial onset sensory and motor neuronopathy (FOSMN) syndrome are presented, one of whom came to a post-mortem examination. This showed TDP-43-positive inclusions in the bulbar and spinal motor neurones as well as in the trigeminal nerve nuclei, consistent with a neurodegenerative pathogenesis. These data support the idea that at least some FOSMN cases fall within the spectrum of the TDP-43 proteinopathies, and represent a focal form of this pathology. S. Karger AG 2015-04-23 /pmc/articles/PMC4448067/ /pubmed/26327906 http://dx.doi.org/10.1159/000381944 Text en Copyright © 2015 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.
repository_type	Open Access Journal
institution_category	Foreign Institution
institution	US National Center for Biotechnology Information
building	NCBI PubMed
collection	Online Access
language	English
format	Online
author	Ziso, Besa Williams, Tim L. Walters, R. Jon L. Jaiser, Stephan R. Attems, Johannes Wieshmann, Udo C. Larner, A.J. Jacob, Anu
spellingShingle	Ziso, Besa Williams, Tim L. Walters, R. Jon L. Jaiser, Stephan R. Attems, Johannes Wieshmann, Udo C. Larner, A.J. Jacob, Anu Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
author_facet	Ziso, Besa Williams, Tim L. Walters, R. Jon L. Jaiser, Stephan R. Attems, Johannes Wieshmann, Udo C. Larner, A.J. Jacob, Anu
author_sort	Ziso, Besa
title	Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
title_short	Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
title_full	Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
title_fullStr	Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
title_full_unstemmed	Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy
title_sort	facial onset sensory and motor neuronopathy: further evidence for a tdp-43 proteinopathy
description	Three patients with the clinical and investigation features of facial onset sensory and motor neuronopathy (FOSMN) syndrome are presented, one of whom came to a post-mortem examination. This showed TDP-43-positive inclusions in the bulbar and spinal motor neurones as well as in the trigeminal nerve nuclei, consistent with a neurodegenerative pathogenesis. These data support the idea that at least some FOSMN cases fall within the spectrum of the TDP-43 proteinopathies, and represent a focal form of this pathology.
publisher	S. Karger AG
publishDate	2015
url	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4448067/
_version_	1613229096736653312

Facial Onset Sensory and Motor Neuronopathy: Further Evidence for a TDP-43 Proteinopathy

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